A 90-year-old woman presented with a 3-month history of an erythematous plaque on the vulva with superficial erosion; it measured about 7×6 cm. A biopsy performed at a nearby hospital revealed an intra-epidermal proliferation of large, pleomorphic, pale cells, distributed singly or in clusters. Most of the tumor cells stained positively with periodic acid Schiff (PAS, pancytokeratin (CAM5.2), and gross cystic disease fluid protein 15 (GCDFP15. She was diagnosed with extramammary Pagetʼs disease and referred to our hospital. Because of her age and poor physical condition, surgery was deemed inappropriate. Because of its relative ease of application, topical imiquimod 5% cream was applied three times weekly to the lesion and immediate perilesional skin. After 3 weeks of treatment, moderate inflammation with erythema and erosion developed in the treated area. The treatment was continued for a total of 6 weeks. One week after completion of the treatment, the lesion appeared to be completely healed. A posttreatment biopsy specimen also revealed resolution of the disease. To our knowledge, only a few cases of extramammary Pagetʼs disease have been reported to be treated successfully with topical imiquimod cream. We consider that topical imiquimod is a promising new treatment option for Pagetʼs disease patients who are not suitable for surgery.